[摘要]目的 探讨法洛四联症(TOF)病儿右心室结构和功能与心肌胶原代谢和基质金属蛋白酶(MMPs)的关系。方法 应用改良BERGMAN法测定23例TOF和18例室间隔缺损(VSD)病儿右心室和右心耳心肌胶原的含量，同时应用RT-PCR技术测定右心室和右心耳心肌组织MMPs mRNA的表达。应用放免法测定血清Ⅲ型胶原氨基末端肽(P-Ⅲ-NP)和Ⅰ型胶原羧基末端肽(P-ⅠCP)的浓度，应用SONOS-5500彩色多普勒超声仪测定TOF病儿右心室内径(RVD)、右心室前壁厚度(RVAW)、右心室舒张早期与舒张晚期峰值血流充盈速度的比值(E/A)。结果 TOF病儿右心室心肌胶原含量、血清P-Ⅲ-NP和P-ⅠCP浓度明显高于VSD组和健康组儿童，右心室心肌组织MMP-1、MMP-2和MMP-9 mRNA的表达较VSD组明显下调。TOF病儿右心室心肌胶原含量、血清P-Ⅲ-NP和P-ⅠCP浓度与E/A呈负相关，与RVD和RVAW呈正相关;TOF病儿右心室心肌组织MMP-1、MMP-2和MMP-9 mRNA的表达与E/A、RVD和RVAW呈正相关。结论 TOF病儿右心室结构和功能与心肌胶原代谢和基质金属蛋白酶的表达密切相关。

    [关键词] 法洛四联症;心肌;胶原;基质金属蛋白酶类

    RELATIONSHIP BETWEEN THE STRUCTURE AND FUNCTION OF RIGHT VENTRICLE AND MATRIX METALLOPROTEINA- SES IN CHILDREN WITH TETRALOGY OF FALLOT

    LI ZI-PU， CAO QIAN， XING QUAN-SHENG

    (Department of Pe- diatrics ， The Affiliated Hospital of Qingdao University Medical College， Qingdao 266003， China)

    [ABSTRACT]ObjectiveTo explore the relationship between the structure and function of right ventricle and matrix metal-loproteinases (MMPs) in children with tetralogy of FALLOT (TOF). MethodsThe contents of myocardial collagen of right ventricle and right auricle were measured in 18 children with ventricular septal defect (VSD) and 23 with TOF using modified Bergman's method; and the myocardial MMPs mRNA expression was determined by RT-PCR. The concentrations of P-Ⅲ-NP and P-ⅠCP were detected by radioimmunoassay. The internal diameter of right ventricle (RV) ......